Dereure Olivier, Jumez Nicolas, Bessis Didier, Gallix Benoit, Guillot Bernard
Department of Dermatology, University of Montpellier I, Hôpital Saint Eloi, Montpellier, France.
Acta Derm Venereol. 2008;88(4):341-5. doi: 10.2340/00015555-0472.
Liver iron content was evaluated by a magnetic resonance imaging-based method in 20 consecutive patients with either sporadic or familial porphyria cutanea tarda. Serum ferritin, hepatitis C infection and the presence of the 2 main mutations of the hemochromatosis gene were also investigated. All patients showed good clinical response to phlebotomy. Initial liver iron content was normal (< 40 micromol/g) in 9 cases, slightly increased (40-59 micromol/g) in 3 cases, moderately increased (60-99 micromol/g) in 6 cases or markedly increased (100-199 micromol/g) in 2 cases). The ferritin level was raised (> 400 ng/ml) in 14/20 patients and there was no obvious relationship with liver iron. Increased liver iron content was observed more frequently in patients with hemochromatosis mutation and less frequent in those with hepatitis C infection. Clinical response to phlebotomies was slightly better in patients with increased liver iron content even slightly, but patients with normal liver iron content also responded well, which suggests that iron depletion is an outstanding treatment independent of liver iron content. This study shows that increased liver iron content is not a constant finding in patients with porphyria cutanea tarda, especially in women, and that it is not a prerequisite for the efficiency of phlebotomy.
采用基于磁共振成像的方法对20例散发性或家族性迟发性皮肤卟啉病患者的肝脏铁含量进行评估。同时还研究了血清铁蛋白、丙型肝炎感染情况以及血色素沉着症基因的2种主要突变的存在情况。所有患者对放血疗法均表现出良好的临床反应。9例患者的初始肝脏铁含量正常(<40微摩尔/克),3例略有升高(40 - 59微摩尔/克),6例中度升高(60 - 99微摩尔/克),2例显著升高(100 - 199微摩尔/克)。20例患者中有14例铁蛋白水平升高(>400纳克/毫升),且与肝脏铁含量无明显关系。血色素沉着症突变患者肝脏铁含量增加更为常见,丙型肝炎感染患者则较少见。即使肝脏铁含量略有增加的患者,放血疗法的临床反应也略好,但肝脏铁含量正常的患者反应也良好,这表明铁耗竭是一种与肝脏铁含量无关的显著治疗方法。本研究表明,肝脏铁含量增加在迟发性皮肤卟啉病患者中并非恒定表现,尤其是在女性患者中,且不是放血疗法疗效的先决条件。